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'Blue Babies' Lost

Managing adult congenital heart disease

Vol. 1 • Issue 1 • Page 27

Due to advances in medical and surgical management of congenital heart disease, the number of Americans surviving into adulthood with it is increasing by about 20,000 each year.1 The U.S. adult congenital heart disease (ACHD) population is now estimated to have reached 1 million, exceeding the pediatric CHD population (see figure).2 But with only 60 ACHD specialist centers in the United States, the healthcare system may be poorly prepared for this huge influx of complex cardiac patients.3 Few primary care providers have the necessary knowledge to manage ACHD patients effectively, and consequently, most ACHD patients either receive inappropriate care or do not have access to care.

Moreover, some CHD patients (and healthcare providers) believe that surgeries performed during childhood have been curative. When many of these patients reach adulthood, they either are discharged from care or are lost to follow-up. Nevertheless, many of the "blue babies" of the 1960s and 1970s now are developing serious long-term complications related to their defect and its repair and require lifelong management.

Compounding the issue is ACHD patients' increased risk for developing serious comorbidities, which require management by primary care providers and those in numerous specialties. Accordingly, PAs and NPs in every specialty are likely to encounter ACHD patients.

state chart

Click to view larger Graphs.

ACHD Background

Concerned about the dearth of knowledge about ACHD and the lack of specialist centers, the American College of Cardiology (ACC) and the American Heart Association (AHA) in 2008 published ACHD management guidelines to assist healthcare providers with clinical decision-making.4 The ACC/AHA guidelines suggest this insufficiency stems in part from the inadequate number of clinicians - including PAs and NPs - with knowledge of ACHD.4 Given the increasing prevalence of ACHD, PAs and NPs should be familiar with the major congenital heart defects, their potential long-term complications and their repair, and be able to refer ACHD patients appropriately.

CHD has challenged medicine for centuries. Affecting about one of every 120 live U.S. births (0.8%), congenital heart defects are the most common birth defect.4 Although CHD's incidence has not changed over time, the medical management of it and its survival rates have changed significantly. At the beginning of the 20th century, most children born with CHD did not survive into adulthood. In 1940, the survival rate was 30%, and most of these survivors had simple defects, such as ventricular and atrial septal defects.4

The development of cardiopulmonary bypass in the 1960s enabled surgeons to repair much more complex defects. By the 1970s, 75% of babies born with CHD survived into adulthood, including some born with more complex defects, such as tetralogy of Fallot and coarctation of the aorta. In the 1980s and 1990s, advances in surgical techniques and critical care medicine further increased survival rates. Today, 90% of infants born with CHD, including those with defects of great complexity such as single ventricle and truncus arteriosus, are expected to survive into adulthood.5

ACHD patients not only are at risk for complications associated with their cardiac disease but also are at increased risk for other diseases. Mackie and colleagues measured ACHD patients' healthcare utilization in their study of 22,096 ACHD patients in Quebec from 1996 to 2000.6 They concluded that ACHD patients have a significantly higher need for primary care than the general population: 68% had visited emergency rooms, 51% had been hospitalized and 16% had been admitted to critical care. And in a 2008 comprehensive review of noncardiac comorbidities in the ACHD population, one expert author concluded that ACHD patients are at increased risk for emergency admission, hepatitis C, scoliosis, diabetes, epilepsy, depression and anxiety.7

Furthermore, in a cross-sectional study of 644 ACHD patients, Pearson and colleagues found 54% to be overweight.8 And a study by Dimopoulos and colleagues suggests that half of all patients with ACHD have significantly higher rates of renal dysfunction.9

The Myth of a 'Cure'

Awareness of the long-term complications and comorbidities associated with ACHD helps dispel the "cure myth" that has long been linked to CHD. Many patients who underwent surgery as children from the 1960s through the 1980s are under the false impression that their defects have been cured and that they need no further follow-up. Many healthcare professionals share this perspective.

In the past, blue babies who underwent surgery in childhood had been predicted to progress to adulthood with few or no complications. Consequently, a large number of children born with CHD either were discharged from care or stopped attending cardiac clinics as they became adults. In adulthood, these "cured" blue babies now are experiencing serious long-term complications, including arrhythmias, valvular dysfunction, heart failure and sudden death. Many of these complications are treatable or even preventable if identified early enough.10

A Complicated Disease

ACHD is an extremely complex condition: With about 35 different defects and multiple treatment methods, patients may present in myriad ways. Some clinicians may be daunted by this complexity, especially those outside the cardiology specialty. To be able to rule out malignant arrhythmias, for example, a clinician must have some knowledge of tetralogy of Fallot, its repair and its long-term complications.

Adding to the complexity are the ever-changing surgical techniques and the lack of ACHD studies. Long-term studies are limited, because open heart surgery on pediatric CHD patients has been possible only since the 1960s. Consider the approach to transposition of the great arteries: In the 1970s and early 1980s, transposition was repaired using an atrial baffle procedure, but now most patients are treated with the arterial switch procedure. The change has occurred because patients who had undergone the atrial baffle procedure now are experiencing serious long-term complications such as malignant arrhythmias, heart failure and sudden death. And the long-term complications of the arterial switch surgery only now are becoming apparent.4

Despite the complexity, ACHD should not be dismissed as beyond a primary care PA or NP's scope. Communication with experts in ACHD and keeping up with treatment protocols are among the ways to become better equipped to manage the care of ACHD patients. The Adult Congenital Heart Association (ACHA) is a trustworthy information resource for providers and patients.11 The ACC/AHA guidelines are available free online and provide a comprehensive survey of approaches to diagnosis and treatment of congenital heart defects.4 This document is based on a meta-analysis of more than 640 congenital heart disease studies and includes a review of the management of long-term complications associated with each defect and its repairs.4 Still, consulting with or referring to an ACHD specialist often is the most appropriate course of action.

Many ACHD patients are neither appropriately referred nor do they receive optimal care, for a variety of reasons. Yeung and colleagues' recent study demonstrates a link between lapses in medical care and adverse outcomes in ACHD patients.12 All of the 158 patients in the study attended an ACHD center and had class II (moderate complexity) or class III (great complexity) defects as defined by ACC/AHA guidelines. The guidelines recommend that patients in these classes visit an ACHD specialist center at least every 2 years. In this study, a lapse of care occurred when that 2-year period was exceeded. Close to two-thirds (63%) of patients met the criteria for a lapse of care. The primary reason was inappropriate advice from their healthcare providers - 32% of these patients were told that cardiac follow-up was not required. Other reasons for lapses in care included patients having been discharged from pediatric care without a follow-up medical facility having been identified (23%); patients dropping out of care, despite advice to the contrary, because they felt well (19%); a lack of insurance (18%); and patient avoidance of care due to fear of receiving bad news (7%).

Indeed, most lapsed patients in the study did receive bad news - 60% received a new diagnosis of hemodynamic significance, such as valvular insufficiency, ventricular dysfunction or pulmonary hypertension. And patients who had lapsed from care had a greater need for urgent cardiac intervention.12 Moreover, given that the study assessed only patients at an ACHD center, it may underestimate the problem's extent.

'Blue Babies' Lost

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I had my first open heart surgery in 1962. I, too, was raised as a "non cardiac-cripple." I did what I wanted in life, but I steered myself toward music and school--law rather than medicine--adopting rathing than pregnancy--having an internal sense of what my body could handle. I have always been followed by a cardiologist. However, I have never been cured of my CHD. And as we age, we do age faster, because our bodies have been through more, before and after the surgery. And I absolutely agree with you--the parents suffer more than children with CHD, because we are strong. But because every case is different, please don't put down people who have worn out earlier than your daughter and are on disability. The symptoms of arryhmias, headaches, pain, lightheadedness after eating, breathing difficulties--these can be present in other CHD's and be stressful and wearing on the body.

Laurie August 31, 2011

As a nurse practitioner and mother of a daughter with tetralogy of Fallt, I was really interested in the "Blue Babies Lost" article. The reason most ACHD patients believe their surgical repair from childhood was/is curative is because that is what they have been told. Fortunately, my daughter has done very well and has a specialist in ACHD patients that she follows up with on a yearly basis. My daughter was born in 1977, never had a BT shunt, and had surgical repair at 20 months of age. Our daughter was never treated differently that other children, participated in sports, and is now pregnant with her third child. Interested research would be on the effect the parental attitude had toward the child's development into adulthood. I am well aware that the surgical repair my daughter had in 1979 is no longer done today, as she had a ventriculostomy. Although I know she does not have a normal heart and am aware of the complciations that still may develop, I feel blessed to have a daughter who has not been made a "cardiac cripple". I know many people with children who are on "disability" for cardiac defects much less critical that a repaired TOF. Having a child with a congenital defect has been challenging. The article did not deal with the psychological process that a parent goes through, but as a parent of such a child I can say there will always be chronic sorrow. What I really want(ed) was a baby with a normal heart, but I would not trade the experience I have had in raising such a wonderful, brave daughter.

Donna Foster,  CFNP,  Nissan Corp. of North American, Canton MSSeptember 17, 2010
Canton, MS


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